RESUMO
Lower urinary tract obstruction in mice can lead to end-stage renal disease and death. We have developed a surgical technique to create a cutaneous vesicostomy in mice providing an external outlet for drainage of urine, thereby relieving the obstruction and slowing and/or preventing the development of end-stage renal disease and death.
Assuntos
Cistostomia/métodos , Obstrução Ureteral/cirurgia , Bexiga Urinária/cirurgia , Derivação Urinária/métodos , Animais , Falência Renal Crônica/prevenção & controle , Masculino , CamundongosRESUMO
Beckwith-Wiedemann syndrome is a rare congenital overgrowth syndrome. It carries important connotations for the pediatric urologist because it is associated with an increased risk for developing certain embryonal tumors. Until recently, these patients were not diagnosed until after birth; however, with newer imaging modalities, this syndrome may be diagnosed prenatally. We present a case of Beckwith-Wiedemann syndrome, depicting the prenatal modalities that may be used to diagnose this unusual condition.
Assuntos
Síndrome de Beckwith-Wiedemann/diagnóstico , Imageamento por Ressonância Magnética , Diagnóstico Pré-Natal/métodos , Ultrassonografia Pré-Natal , Adulto , Feminino , Humanos , GravidezRESUMO
Congenital obstructive nephropathy (CON) is the most common cause of chronic renal failure in children often leading to end-stage renal disease. The megabladder (mgb) mouse exhibits signs of urinary tract obstruction in utero resulting in the development of hydroureteronephrosis and progressive renal failure after birth. This study examined the development of progressive renal injury in homozygous mgb mice (mgb-/-). Renal ultrasound was used to stratify the disease state of mgb-/- mice, whereas surgical rescue was performed using vesicostomy. The progression of renal injury was characterized using a series of pathogenic markers including alpha smooth muscle isoactin (α-SMA), TGF-ß1, connective tissue growth factor (CTGF), E-cadherin, F4/80, Wilm's tumor (WT)-1, and paired box gene (Pax) 2. This analysis indicated that mgb-/- mice are born with pathologic changes in kidney development that progressively worsen in direct correlation with the severity of hydronephrosis. The initiation and pattern of fibrotic development observed in mgb-/- kidneys appeared distinctive from previous animal models of obstruction. These observations suggest that the mgb mouse represents a unique small animal model for the study of CON.
Assuntos
Hidronefrose/congênito , Hidronefrose/patologia , Falência Renal Crônica/congênito , Falência Renal Crônica/patologia , Rim/lesões , Nefrite Intersticial/congênito , Nefrite Intersticial/patologia , Animais , Criança , Cistostomia , Modelos Animais de Doenças , Progressão da Doença , Fibrose , Humanos , Hidronefrose/complicações , Hidronefrose/cirurgia , Rim/diagnóstico por imagem , Rim/patologia , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Masculino , Camundongos , Camundongos Knockout , Camundongos Mutantes , Nefrite Intersticial/complicações , Nefrite Intersticial/cirurgia , UltrassonografiaRESUMO
Urethral duplication is an uncommon clinical finding, and classification systems have been described to explain the clinical findings. Urethral triplication is an extremely uncommon diagnosis, with few published case reports. We present the rare case of an infant noted to have a bifid urinary stream and found to have urethral triplication on voiding cystourethrography.